Chiari type III malformation associated with Klippel-Feil syndrome, a case report with a narrative review of the literature.

BACKGROUND: Chiari malformation type III (CM III), a rare hindbrain anomaly, often presents with various concurrent anomalies. This paper reports a unique case of CM III associated with Klippel-Feil syndrome (KFS), a condition previously unreported in Saudi Arabia and documented in only one other case globally in Turkey. This study aims to share insights into the unusual association between CM III and KFS, considering their close embryological development and involvement in the craniocervical junction. METHODOLOGY: The study presents a case of a 2.5-year-old female diagnosed with CM III and KFS. Diagnostic tools such as ultrasound, CT scans, MRI, and physical examinations were used to confirm the patient's condition. Surgical interventions, including decompression and encephalocele repair, were performed. RESULTS: Successful surgical interventions, including encephalocele repair and duraplasty, were carried out. Follow-up visits indicated a stable condition, marked improvement in lower limb strength, and the patient's ability to walk with assistance. CT follow-up affirmed a satisfactory surgical outcome. CONCLUSION: This case study illustrates the potential for an optimistic prognosis in CM III, even when accompanied by complex conditions such as KFS, through early diagnosis and intervention. It underscores the significance of antenatal screening for effective care planning and calls for further research and publications due to the rarity of this association. These findings contribute to our understanding of CM III and its related conditions, emphasizing the need for open-minded consideration of potential embryological associations.

Complications and visual outcomes following surgical resection of pediatric optic pathway/hypothalamic gliomas: a systematic review and meta-analysis.

Pediatric optic pathway/hypothalamic gliomas (OPHG) pose challenges in treatment due to their location and proximity to vital structures. Surgical resection plays a key role in the management of OPHG especially when the tumor exhibits mass effect and causes symptoms. However, data regarding outcomes and complications of surgical resection for OPHG remains heterogenous. The authors performed a systematic review on pediatric OPHG in four databases: PubMed, EMBASE, Cochrane Library, and Google Scholar. We included studies that reported on the visual outcomes and complications of OPHG resection. A meta-analysis was performed and reported per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A total of 26 retrospective studies were included. Seven hundred ninety-seven pediatric patients with OPHG undergoing surgical resection were examined. A diagnosis of NF1 was confirmed in 9.7%. Gross total resection was achieved in 36.7%. Intraorbital optic pathway gliomas showed a significantly higher gross total resection rate compared to those located in the chiasmatic/hypothalamic region (75.8% vs. 9.6%). Postoperatively, visual acuity improved in 24.6%, remained unchanged in 68.2%, and worsened in 18.2%. Complications included hydrocephalus (35.4%), anterior pituitary dysfunction (19.6%), and transient diabetes insipidus (29%). Tumor progression post-resection occurred in 12.8%, through a mean follow-up of 53.5 months. Surgical resection remains an essential strategy for treating symptomatic and large pediatric OPHG and can result in favorable vision outcomes in most patients. Careful patient selection is critical. Patients should be monitored for hydrocephalus development postoperatively and followed up to assess for tumor progression and adjuvant treatment necessity.

Dynamic interplay between corticosteroid treatment and the role of SRC-1 gene dysregulation in the progression of WHO-Grade 4 Astrocytoma.

BACKGROUND: Corticosteroid is commonly used before surgery to control cerebral oedema in brain tumours and is frequently continued throughout treatment. Its long-term effect of on the recurrence of WHO-Grade 4 astrocytoma remains controversial. The interaction between corticosteroid, SRC-1 gene and cytotoxic T-cells has never been investigated. METHODS: A retrospective cohort of 36 patients with WHO-Grade 4 astrocytoma were examined for CD8 + T-cell and SRC-1 gene expressions through IHC and qRT-PCR. The impact of corticosteroid on CD8+T-cells infiltration, SRC-1 expression, and tumour recurrence was analyzed. RESULTS: The mean patients age was 47-years, with a male to female ratio 1.2. About 78% [n = 28] of the cases showed reduced or no CD8+T-cell expression while 22% [n = 8] of cases have showed medium to high CD8+T-cell expression. SRC-1 gene was upregulated in 5 cases [14%] and 31 cases [86%] showed SRC-1 downregulation. The average of total days and doses of administered corticosteroid from the preoperative period to the postoperative period was at range of 14-106 days and 41-5028 mg, respectively. There was no significant statistical difference in RFI among tumours expressing high or low CD8+T-cells when corticosteroid was administered in recommended or exceeded doses [p-value = 0.640]. There was a significant statistical difference in RFI between CD8+T-Cell expression and SRC-1 gene dysregulation [p-value = 002]. Tumours with high CD8+T T-cell expression and SRC-1 gene downregulation had late recurrence. CONCLUSIONS: Corticosteroid treatment can directly affect the SRC-1 gene regulation but does not directly influence cytotoxic T-cells infiltration or tumor progression. However, SRC-1 gene downregulation can facilitate late tumor recurrence.

Outcomes after laser interstitial thermal ablation for temporal lobe epilepsy: a systematic review and meta-analysis.

Epilepsy is a common condition that affects approximately 1% of the world's population, with about one-third being refractory epilepsy. Temporal lobe epilepsy is the most common type of drug-resistant epilepsy, and laser interstitial thermal therapy (LITT) is an innovative treatment. In this systematic review and meta-analysis, we aimed to summarize the current evidence on outcomes after LITT, including seizure freedom rate, complication rate, and neurocognitive outcome. PubMed and OVID Medline search engines were systematically searched for all indexed publications in the English language up to July15, 2023. The search was limited to human studies. Proportions and 95% confidence interval (CI) values were calculated for seizure, neurocognitive outcome, and complication rate. A total of 836 patients were included. Overall seizure outcomes, regardless of the pathology, included Engel I outcome in 56% (95% CI, 52.4-59.5%), Engel II outcome in 19.2% (95% CI, 15.4-23.6%), Engel III outcome in 17.3% (95% CI, 13.5-21.8%), and Engel IV outcome in 10.5% (95% CI 6.3-17%) of the patients. The overall decline in verbal and visual memory regardless of laterality was 24.2 (95% CI 8.6-52%) and 25.2% (8.3-55.8%). For naming, the decline was 13.4% (6.6-25.4%). The results of the pooled analysis in comparison with available data in the literature showed that seizure outcomes after LITT were slightly inferior to published data after temporal lobectomy. Data on cognitive outcomes after LITT are scarce and heterogeneous.

Ventriculoperitoneal shunt-associated abdominal cerebrospinal fluid pseudocysts and the role of laparoscopy and a proposed management algorithm in its treatment. A report of 2 cases.

Abdominal cerebrospinal fluid pseudocyst (APC) is a rare complication associated with a ventriculoperitoneal shunt (VPS) placement. Abdominal cerebrospinal fluid pseudocyst is defined as a cyst surrounded by a non-epithelial lining and filled with the cerebrospinal fluid (CSF). In general, 1% - 4.5% of all patients treated with a VPS develop CSF pseudocysts. Here, we have presented 2 cases of APC complicating VPS placement and a proposed treatment management algorithm. The cases pertained to 7-year-old and 5-year-old children who had undergone VPS placement during infancy and presented with progressive abdominal distention and vomiting and fever, respectively. Both cases were assessed using similar investigations and imaging modalities and diagnosed with APC. However, the treatments were individually tailored based on the algorithm; successful outcomes were achieved in both patients. Abdominal cerebrospinal fluid pseudocyst treatment is difficult considering the presence of adhesions and infection and is associated with a high recurrence rate.

Electrocardiographic changes following intracranial haemorrhage: a retrospective cohort study.

OBJECTIVE: To improve the understanding of the association between electrocardiographic (ECG) abnormalities and clinical outcomes of patients with all types of intracranial hemorrhage (ICH). METHODS: A retrospective cohort study was conducted in a tertiary healthcare hospital on patients with ICH without cardiac disease or renal disease requiring dialysis. Demographic and clinical data were collected from hospital records. ECG record were obtained within 24 hours of presentation and prior to treatment. Records were interpreted for this study by a cardiologist blinded to other data. RESULTS: Assessment of 291 patients (228 adults and 63 children) showed that subdural hemorrhage was the most common type of ICH (31.6%) followed by intraparenchymal hemorrhage (23.0%). ECG records were available for 98 patients. ECG abnormalities were most commonly nonspecific ST-segment changes (32.6%). In adults, history of neurological disease was associated with atrioventricular block (p=.004) and QTc prolongation (p=.041). Pediatric patients exhibited associations between ST-segment changes (p=.045) and sinus tachycardia (p=.027) and type of ICH. However, ECG changes were not statistically associated with clinical outcomes in adults or children. CONCLUSION: Significant ECG changes frequently occurred in patients with all types of ICH but did not consistently predict the outcome in this study. Close observation of patients is still recommended to detect ECG changes that could affect the treatment.

Evaluating the initial impact of the Riyadh Epilepsy Awareness Campaign.

OBJECTIVE: We determine the impact of an educational awareness campaign on the level of knowledge and the attitude of the Saudi population in Riyadh, Saudi Arabia in an attempt to improve the awareness and reduce the social stigma associated with epilepsy. METHODS: The Saudi Epilepsy Society organized a citywide awareness campaign in 2013. A survey consisting of 11 questions pertaining to epilepsy awareness was distributed to Saudi citizens living in Riyadh, aged 15 years and above, in malls, health clubs, mosques, universities, and schools during that campaign. The same questionnaire was administered before and after the awareness campaign to the same individuals on the same day to assess the impact of the campaign (n=2118). RESULTS: The epilepsy awareness campaign significantly raised the general knowledge about epilepsy: 1519 before vs. 1944 after (P<0.001) would allow their children to interact with an individual who had epilepsy; 1567 before vs. 688 after (P<0.001) would not want their children to marry an individual with epilepsy. Eight hundred twenty six before vs. 47 after (P<0.001) thought that epilepsy is untreatable. Regarding the causes of epilepsy, 1663 before vs. 896 after (P<0.001) believed that epilepsy is caused by supernatural powers, and 1224 before vs. 1874 after (P<0.001) chose brain disease as a cause of epilepsy. CONCLUSION: These findings suggest that epilepsy awareness campaigns can close knowledge gaps. A long-term reevaluation may be needed to assess awareness sustainability.

Focal brainstem gliomas. Advances in intra-operative management.

Improved neuronavigation guidance as well as intraoperative imaging and neurophysiologic monitoring technologies have enhanced the ability of neurosurgeons to resect focal brainstem gliomas. In contrast, diffuse brainstem gliomas are considered to be inoperable lesions. This article is a continuation of an article that discussed brainstem glioma diagnostics, imaging, and classification. Here, we address open surgical treatment of and approaches to focal, dorsally exophytic, and cervicomedullary brainstem gliomas. Intraoperative neuronavigation, intraoperative neurophysiologic monitoring, as well as intraoperative imaging are discussed as adjunctive measures to help render these procedures safer, more acute, and closer to achieving surgical goals.

Pediatric brainstem tumors. Classifications, investigations, and growth patterns.

Brainstem gliomas occur in 10-20% of brain tumors in pediatrics. Over the past 3 decades, the treatment of brainstem gliomas has significantly progressed as a result of the gradual advancements in microsurgical techniques, sophisticated imaging technology and, most importantly, the availability of MRI. In this article, we review the current literature on brainstem gliomas and cover diagnosis, imaging, classification, and management. Surgical approaches and intraoperative modalities to tackle operable cases of brainstem gliomas will be discussed in a follow up article.